Usual and Unusual Manifestations of Familial Hemophagocytic Lymphohistiocytosis and Langerhans Cell Histiocytosis

Pediatr Clin North Am. 2017 Feb;64(1):91-109. doi: 10.1016/j.pcl.2016.08.006.

Abstract

Familial hemophagocytic lymphohistiocytosis (FHL) and Langerhans cell histiocytosis (LCH) are histiocytic diseases that occur most commonly in young children. Improvements in recognition and treatment have been substantial for both diseases in the past decade, although early and late morbidity continue to be major concerns. These two diagnoses behave differently, although the clinical spectra for both diseases are diverse and can lead to confusion and delays in diagnosis and treatment. This article focuses on the clinical and genetic spectrum of FHL as well as the clinical and treatment variations of LCH.

Keywords: Familial hemophagocytic lymphohistiocytosis (FHL); Hemophagocytic lymphohistiocytosis (HLH); Histiocytic disorders; Langerhans cell histiocytosis (LCH).

Publication types

  • Review

MeSH terms

  • Child
  • Delayed Diagnosis
  • Diagnosis, Differential
  • Diagnostic Errors
  • Histiocytosis, Langerhans-Cell / diagnosis*
  • Histiocytosis, Langerhans-Cell / genetics
  • Histiocytosis, Langerhans-Cell / therapy
  • Humans
  • Lymphohistiocytosis, Hemophagocytic / diagnosis*
  • Lymphohistiocytosis, Hemophagocytic / genetics
  • Lymphohistiocytosis, Hemophagocytic / therapy
  • Rare Diseases / diagnosis*